This article is based on a report presented at the Second International Conference on Cholesteatoma and Mastoid Surgery, in Tel Aviv, in March 1981. The principal aim is to illustrate our personal experience with the so-called congential cholesteatoma of the middle ear, that is, a cholesteatoma growing inside the tympanic cavity affecting subjects with no inflammatory processes in their clinical history and a perfectly normal eardrum. Eleven cases out of a toal of 429 cases with cholesteatoma (2.56%) operated on at the Otorhinolaryngology Clinic II of Parma are reported. All patients—nine children and two adults—underwent a combined-approach closed tympanoplasty. The diagnostic stages are analyzed in particular, as well as the clinical and impedance audiometric data, which are correlated with surgical findings. Pathogenetic mechanisms are discussed briefly. The need for screenings to be carried out in schools and an accurate evaluation of all unilateral impairments in hearing conduction are emphasized with a view to early diagnosis.